Goodpasture syndrome

Goodpasture syndrome is a kidney and lung disease caused by autoantibodies directed against collagen components of the basement membrane. While in the kidney the disease causes progressive renal failure lung bleeding may even cause an emergency. Sometimes the disease is observed in patients with Alport syndrome after successful transplantation.

Diagnosis

The diagnosis is made clinically by the conjunction of kidney (proteinuria, glomerular hematuria and progressive renal failure) and pulmonary (dyspnea and hemoptysis) symptoms. The diagnosis is proved if autoantibodies against glomerular basement membrane are detected.

Pathogenesis

Though Goodpasture syndrome is an acquired disease, it often runs in families, among twins in particular. Association was found with the MHC locus which is not surprising as Goodpasture syndrome is an immunological disorder.

Biochemical studies revealed autoantibodies directed against particular regions of the NC1 domain of the collagen 4 chains 3 and 5, genes COL4A3 and COL4A5 respectively. Alport patients who develop Goodpasture after receiving a renal transplant show a broader spectrum of antibodies.

How a serine/threonine kinase that binds to the same region may be involved in the pathogenesis is yet to be elucidated.